Optic pathway glioma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
Optic pathway glioma (OPG) is a benign tumor that develop along the optic nerve (chiasm, tracts, and radiations) characterized by impairment or loss of vision and may be accompanied by diencephalic symptoms such as reduced growth and alteration in sleeping patterns. OPG are often linked to neurofibromatosis type 1 (NF1).
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Optic pathway glioma | |
| fr | Gliome des voies optiques | |
| de | Optikusgliom | |
| es | Glioma de vías ópticas | |
| it | Glioma delle vie ottiche | |
| nl | Optische banenglioom | |
| pt | Glioma das vias ópticas | |
| pl | Glejak nerwu wzrokowego | |
| cs | Gliom optické dráhy | |
| uk | Гліома зорового тракту | |
| zh | 视路胶质瘤 | |
| tr | Optik yolak gliomu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D33.3 | NTBT | attributed | validated |
| ICD-11 | 2A02.0Y | NTBT | index | validated |
| MONDO | 0016167 | E | validated | |
| UMLS | C0796418 | E | validated | |
| GARD | 4107 | E | validated | |
| MedDRA | 10073338 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (2):
- ORPHA:95503Pituitary hormone deficiency of tumoral origin
rare endocrine diseases - ORPHA:252057Tumor of cranial and spinal nerves
rare neurological diseases; rare neoplastic diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.12 / 100 000 | Worldwide | Value and class | Not yet validated | 22796286[PMID] |
| Point prevalence | Unknown | — | Europe | Class only | Not yet validated | ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
23 annotations · validated 2019-05-16| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Frequent (79-30%) | ||
| HP:0001067 | Neurofibromas | |
| HP:0000639 | Nystagmus | |
| HP:0000648 | Optic atrophy | |
| HP:0007663 | Reduced visual acuity | |
| HP:0000572 | Visual loss | |
| Occasional (29-5%) | ||
| HP:0000707 | Abnormality of the nervous system | |
| HP:0000618 | Blindness | |
| HP:0002376 | Developmental regression | |
| HP:0003473 | Fatigable weakness | |
| HP:0001263 | Global developmental delay | |
| HP:0001510 | Growth delay | |
| HP:0002315 | Headache | |
| HP:0000238 | Hydrocephalus | |
| HP:0002018 | Nausea | |
| HP:0000602 | Ophthalmoplegia | |
| HP:0001085 | Papilledema | |
| HP:0000826 | Precocious puberty | |
| HP:0000520 | Proptosis | |
| HP:0001250 | Seizure | |
| HP:0000486 | Strabismus | |
| HP:0002321 | Vertigo | |
| HP:0001123 | Visual field defect | |
| HP:0002013 | Vomiting | |
Annotation source: 27082859[PMID]_28847625[PMID]_28984541[PMID]_29246098[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/2086
- This record as JSON: /data/d/2086.json